Management of pulmonary arterial hypertension associated to thalassemia: when pulmonary endarterectomy is the best therapeutical option? A case report

Pulmonary arterial hypertension (PAH) has been reported with nearly all forms of the inherited as well as the acquired hemolytic anemias. Although screening studies suggested that PAH has emerged as major complication of thalassemia patients, its impact on survival is unknown; the pathophysiology of the PAH in these patients is multifactorial, and a thorough diagnostic evaluation is essential. Understanding the PAH pathogenesis, diagnostic options, prevention is critical for clinicians who care for the thalassemic patients; there are virtually no high-quality data on the safety/efficacy of PAH treatment strategy in this patient population. We are reporting the case of a thalassemic patient suffering from progressive severe PAH, not responding to medical treatment and related to chronic thromboembolic disease. After carefully considering all the options, we decided to proceed with vascular disobliteration by pulmonary endarterectomy (PEA), the first line choice in these cases. This intervention led to a significant improvement in the clinical status and in the functional parameters. Therefore, even if haemolytic anemia-associated-PAH is included in the group I of the Dana-point classification, an individualized approach is recommended as well as a particular management with disease-specific measures and a comprehensive evaluation of other causes of PAH; this current report supports the feasibility and effectiveness of PEA also in the thalassemic patients with surgically accessible chronic thromboembolic pulmonary hypertension.